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PLoS One. 2016 Jul 6;11(7):e0156679. doi: 10.1371/journal.pone.0156679. eCollection 2016.

Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

Author information

1
Service d'Hématologie, Hôpital Saint-Antoine, AP-HP, Paris, France.
2
Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, AP-HP, et UPMC Univ Paris 06, Paris, France.
3
Service de Médecine interne, CHU de Fort-de France, Fort-de-France, Martinique, France.
4
AP-HP, Laboratoire Jean Dausset d'Immunologie et d'Histocompatibilité & INSERM, UMRS 1160, Hôpital Saint Louis, Sorbonne Paris Cité, Paris, France.
5
Université Paris Diderot, Paris, France.
6
Service de Réanimation Médicale, Hôpital Saint-Louis, AP-HP, Paris, France.
7
Service d'Immunologie clinique, Hôpital Saint-Louis, AP-HP, Paris, France.
8
Service de Réanimation Médicale, Hôpital Cochin, AP-HP, Paris, France.
9
Université Paris 5, Paris, France.
10
Service de Néphrologie, Hôpital Albert Calmette, Lille, France.
11
Service de Médecine Interne, Hôpital Bichat, AP-HP, Paris, France.
12
Service de Médecine interne 1, Hôpital La Pitié-Salpêtrière, AP-HP, Paris, France.
13
Service d'Hématologie Biologique, Hôpital Lariboisière, AP-HP, Paris, France.
14
Sorbonne Université, UPMC Univ Paris 06, Paris, France.
15
Inserm U1009, Institut Gustave Roussy, Villejuif, France.

Abstract

Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all). Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03). Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

PMID:
27383202
PMCID:
PMC4934773
DOI:
10.1371/journal.pone.0156679
[Indexed for MEDLINE]
Free PMC Article

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